硬纤维瘤最初不可切除-约2例

Sara Senti Farraraons, A. González, C. Velazquez, Clara Gené Škrabec, I. C. Ausàs
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摘要

硬纤维瘤,也称为侵袭性纤维瘤病,是一种罕见的结缔组织和骨骼肌腱膜的纤维母细胞增生。硬纤维瘤的病因尚不清楚,但它们与雌激素刺激、既往创伤、手术干预和妊娠有关。虽然它的特点是没有转移或分化的潜力,但它是一种非包被性病变,具有浸润性生长和不可预测的行为。因此,与其他低程度恶性肉瘤相比,它可能具有侵袭性,也可能是非常惰性的,甚至会自发消退。在过去的几年里,硬纤维瘤的治疗选择已经发生了变化,因为无症状患者的术前手术已经被主动监测所取代。然而,在某些特殊情况下,如腹内病变,治疗必须个体化。我们提出了两例患者诊断腹腔内硬纤维瘤的威胁行为,治疗方法和最终决议。
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Desmoid tumor initially unresectable – About two cases
Desmoid tumor, also called aggressive fibromatosis, is a rare fibroblastic proliferation of connective tissue and skeletal muscle aponeurosis. The aetiology of desmoid tumours is poorly understood, but they have been related with oestrogen stimuli, previous trauma, surgical interventions and pregnancy. Although it is characterized by not having potential for metastasize or differentiate, it is a non-encapsulated lesion, with an infiltrative growth and unpredictable behaviour. In this way, it could have either an aggressive pattern, when compared with other low degree malignancy sarcomas, or a very indolent one, even with spontaneous regression. Therapeutic options in desmoid tumours have been changing over these last years, as surgery upfront in asymptomatic patients has been superseded by active surveillance. However, treatment has to be individualized in some specific scenarios such as intra-abdominal lesions. We present two cases of patients diagnosed with an intra-abdominal desmoid tumour with threatening behaviour, the treatment approach and final resolution.
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